RESUMO
Pulmonary arterial hypertension is a severe disease with a complex pathogenesis, for which combination therapy is an attractive option.This study aimed to assess the impact of sequential combination therapy on both short-term responses and long-term outcomes in a real-world setting.Patients with idiopathic/heritable pulmonary arterial hypertension, or pulmonary arterial hypertension associated with congenital heart disease or connective tissue disease and who were not meeting treatment goals on either first-line bosentan or sildenafil monotherapy, were given additional sildenafil or bosentan and assessed after 3-4 months. Double combination therapy significantly improved clinical and haemodynamic parameters, independent of aetiology or the order of drug administration. Significant improvements in functional class were observed in patients with idiopathic/heritable pulmonary arterial hypertension. The 1-, 3- and 5-year overall survival estimates were 91%, 69% and 59%, respectively. Patients with pulmonary arterial hypertension associated with connective tissue disease had significantly poorer survival rates compared to other aetiologies (p<0.003).The favourable short-term haemodynamic results and good survival rates, observed in patients receiving both bosentan and sildenafil, supports the use of sequential combination therapy in patients failing on monotherapy in a real-world setting.
Assuntos
Anti-Hipertensivos/administração & dosagem , Hipertensão Pulmonar Primária Familiar/complicações , Hipertensão Pulmonar/tratamento farmacológico , Citrato de Sildenafila/administração & dosagem , Sulfonamidas/administração & dosagem , Vasodilatadores/administração & dosagem , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Anti-Hipertensivos/uso terapêutico , Bosentana , Causas de Morte , Criança , Doenças do Tecido Conjuntivo/complicações , Quimioterapia Combinada , Teste de Esforço , Feminino , Cardiopatias Congênitas/complicações , Hemodinâmica/efeitos dos fármacos , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/mortalidade , Itália , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Citrato de Sildenafila/uso terapêutico , Sulfonamidas/uso terapêutico , Taxa de Sobrevida , Resultado do Tratamento , Vasodilatadores/uso terapêutico , Adulto JovemRESUMO
BACKGROUND: Integrated transthoracic and transesophageal echocardiography enables identification and characterization of a quadricuspid aortic valve anomaly. CASE PRESENTATION: A totally asymptomatic 40-year-old white man was referred to our Division of Cardiology after accidental finding of a heart murmur. Transesophageal echocardiography detected a quadricuspid aortic valve characterized by four cusps of equal size and severe aortic valvular regurgitation, without any further anomalies. He underwent a successful aortic valve repair. CONCLUSIONS: Quadricuspid aortic valve anomaly is a rare congenital cardiac defect that can cause progressive valvular complications.
Assuntos
Insuficiência da Valva Aórtica/diagnóstico por imagem , Valva Aórtica/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico por imagem , Doenças das Valvas Cardíacas/diagnóstico por imagem , Adulto , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/cirurgia , Ecocardiografia Transesofagiana , Cardiopatias Congênitas/cirurgia , Doenças das Valvas Cardíacas/cirurgia , Humanos , MasculinoRESUMO
BACKGROUND: The primary objective of this post hoc analysis was to evaluate clinical outcomes of tadalafil in patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD-PAH) compared with patients with idiopathic/heritable PAH (I/H-PAH) for primary and key secondary efficacy endpoints, and safety. This analysis included adult patients with CTD-PAH or I/H-PAH who participated in the PHIRST and PHIRST-2 studies. METHODS: Patients were randomized 1:1:1:1:1 to tadalafil (2.5, 10, 20, or 40mg) or placebo in the PHIRST study and the majority of these patients were subsequently assigned 40mg in PHIRST-2. Patients taking 20mg in PHIRST without demonstrating clinical worsening continued on 20mg in PHIRST-2. Outcomes analyzed included 6MWD, WHO-FC, and incidence and time to first occurrence of clinical worsening. Safety was assessed through evaluation of adverse events (AEs), clinical laboratory data, electrocardiograms, and physical examinations. RESULTS: Increased 6MWD in PHIRST was maintained in both CTD-PAH and I/H-PAH subgroups for 52weeks. Patients with CTD-PAH tended to be older, were more likely female, had lower exercise capacity, were more likely to have clinical worsening, and experienced AEs more frequently than patients with I/H-PAH. CONCLUSION: The effect of tadalafil treatment in patients enrolled in both PHIRST studies was detectable for both I/H-PAH and CTD-PAH subgroups. In general, subgroup differences were modest. Patients with CTD-PAH may perform less well than patients with I/H-PAH in safety and efficacy measures in all treatment groups, which is similar to other studies demonstrating a worse prognosis for patients with CTD-PAH.